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DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR

Contrast-enhanced CT T1W MR w/Gadolinium Proton Density MR
DYSEMBRYOPLASTIC NEUROEPITHELIAL TUMOR

WHO Grade I

Cell of Origin: Histogenesis uncertain. Hypothesized to arise from external granular layer of the cortex.

Synonyms: DNET or DNT

Common Locations: Cortical, temporal lobe

Demographics: May be seen at any age; most common in children and young adults

Clinical Presentation: Partial complex seizures, especially beginning before age 20

Histology: Diagnosis based on presence of: 1) a specific glioneuronal element, consisting of oligodendrocytes in a mucinous matrix in which neurons appear to "float," or 2) glial nodules associated with cortical dysplasia.

Special Stains: Neuronal markers (synaptophysin, neuronal specific enolase) and glial markers (GFAP, S-100) positive. Despite benign behavior, may have high MIB-1 labeling index

Progression: Usually stable, even over long periods.

Radiology: Nodular cortical lesion without edema or mass effect. May have megagyric or multicystic appearance. Occasionally may enhance or contain calcifications. CT may show calvarial remodeling.

Comments: Surgically curable cause of seizures.